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WHO Classification of Tumours
Sebaceous adenocarcinoma
Vulva


Definition

Sebaceous carcinoma (SC) is a malignant adenocarcinoma with sebaceous differentiation. The lesion typically arises in the ocular adnexae and in the skin of head and neck, trunk, genitals, and extremities. SC presents as a painless, typically slow-growing, nodular mass and may be multifocal. Histologically, the tumour is characterized by dermal lobules of malignant sebocytic cells with multivesicular and vacuolated clear cytoplasm, surrounded by a fibrovascular stroma. The centre of the tumour cell nests may be necrotic. SCs may be associated with overlying carcinoma in-situ or with extramammary Paget disease, or both, in the surface epithelium and in other epidermal appendages.
Sebaceous carcinoma usually arises in middle-aged to elderly patients (average age 62 years). Women are twice as frequently affected. The risk for local recurrence is 30-40%, the risk for distant metastases 20-25%. Tumour-related mortality is 10-20%

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Moreno C, Jacyk WK, Judd MJ, Requena L (2001)
Highly aggressive extraocular sebaceous carcinoma.
Am J Dermatopathol 23: 450-5




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LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



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Despite numerous sebaceous glands present on the vulva, vulvar sebaceous carcinoma is rare. The tumour resembles its cutaneous counterpart. It is composed of cords and nests of basaloid appearing neoplastic glandular elements with cellular features of sebaceous epithelium. It may be associated with vulvar intraepithelial neoplasia (VIN)

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Escalonilla P, Grilli R, Cañamero M, Soriano ML, Fariña MC, Manzarbeitia F, Sáinz R, Matsukura T, Requena L (1999)
Sebaceous carcinoma of the vulva.
Am J Dermatopathol 21: 468-72




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Carlson JW, McGlennen RC, Gomez R, Longbella C, Carter J, Carson LF (1996)
Sebaceous carcinoma of the vulva: a case report and review of the literature.
Gynecol Oncol 60: 489-91




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Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



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